Blistering Skin Disorders

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Definition

Bullous Pemphigoid

Pemphigoid is a rare blistering disorder, which usually occurs in later life. The blisters come up on the skin and, less often, in the mouth too. Pemphigoid is classed as an autoimmune disease. For an unknown reason, antibodies form in the blood and then attack the structures holding the outer layer of the skin onto the deeper layers. This leads to splitting of the skin and so into blisters. An itchy rash may be present for some weeks before any blisters come up. The blisters have thick roofs, and can get quite large and tense before they burst. Most contain clear fluid but in a few this is bloodstained. Your doctor may perform a skin biopsy and special tests (immunofluorescence) to confirm the diagnosis.

Pemphigus Vulgaris

Pemphigus vulgaris is a rare autoimmune skin disease. Pemphigus vulgaris may cause severe blistering of the skin and the mucous membranes lining the mouth, nose, throat and genital area. Blisters develop in the upper layer of the skin, and have a thin and fragile outer surface that breaks away easily to leave raw areas (erosions) that can be extensive and painful. Pemphigus vulgaris does not go away by itself, and always needs assessment and treatment supervised by a Dermatologist. The immune system makes antibodies (auto antibodies) that attack the skin and mucous membrane leading to damage that causes blisters to develop. Your doctor may perform a skin biopsy and special tests (immunofluorescence) to confirm the diagnosis.

Staphylococcal Scalded Skin Syndrome

Staphylococcal scalded skin syndrome is a widespread painful rash caused by bacteria called Staphylococcus aureus, which produces a toxin that damages the outer layer of the skin causing it to be shed. The skin then resembles a scald or burn. Affected patients are miserable, feverish and may not want to be held or touched.

Treatment and drugs

Bullous Pemphigoid and Pemphigus Vulgaris

Treatment has three aims – to stop new blisters coming up; to heal the blisters that are already there; and to use the smallest possible doses of the medicines, as side effects can be common and severe. A steroid cream can help if only a small area of skin is affected. Antibiotics called tetracyclines, sometimes combined with nicotinamide, may help in Bullous Pemphigoid and can be combined with other treatments. Treatment for more severe blistering is usually with high doses of steroid tablets to get the blistering under control quickly. Other tablets that affect the immune system can be used at the same time as the steroid tablets, the aim being to reduce the steroid dose. Examples of additional tablets that can be used in this way are azathioprine, cyclosporine, methotrexate and, dapsone. However, they can all cause side effects too, and their use has to be considered carefully.

Staphylococcal Scalded Skin Syndrome

Usually antibiotics will need to be given intravenously. At the same time as having antibiotics, the skin needs to be gently cleansed with a soap substitute, which may contain an antiseptic. Greasy moisturisers are recommended in order to soothe the skin and to stop the healing skin from sticking to clothing or bedding.